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Q. I am 18 years old and diagnosed with PAPVC. How can I fix it?

Answered by
Dr. Sagar Ramesh Makode
and medically reviewed by iCliniq medical review team.
This is a premium question & answer published on Jun 16, 2018

Hi doctor,

I am a 18-year-old girl who was diagnosed with a PAPVC defect on the left upper pulmonary vein seven months back and have had symptoms for quite a while like poor circulation, exercise intolerance which seems to get worse even as I gradually try to build up endurance and my current cardiologist does not. I should explore the idea of fixing it even though my MRI shows that even with just one vein my blood ratio on one side to the other is noticeable and borderline. I have an appointment with a electrophysiology because I seem to be tachycardic quite often even though my pulse used to air in the 70’s also because I have been having episodes they think could be an arrhythmia and possibly related to the defect.

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#

Hello,

Welcome to icliniq.com.

Do you get frequent respiratory tract infection like pneumonia since your childhood? Please upload MRI and echo reports. Is your hemoglobin fine? Usually, with single vein shunt is not significant, and severity of shunt can be seen on echo as well. So in small defect surgery is not considered unless shunt ratio is more than 2:1. As there is no effect of surgery on survival so it is not done. So if reports are not showing any evidence of damage to heart then surgery may not be done. However, you should be given a trial of low dose diuretics like tablet Torsemide 5 to 10 mg and look for a response in term of exercise tolerance. Hemoglobin should be corrected if it is low at it can cause these symptoms. You should try to exercise and gradually increase the exercise without thinking about the defect.

Yes, a cardiac arrhythmia may be associated with the defect. Since you are having frequent palpitations, you should have Holter monitoring done, which is 24 ambulatory ECG monitoring, which will detect the rhythm during palpitations and confirm whether arrhythmias is there or not. In treatment part, you should be given beta blockers like Metoprolol after we rule out or rule in diagnosis for palpitations.

For more information consult a cardiologist online --> https://www.icliniq.com/ask-a-doctor-online/cardiologist

Thank you doctor,

I will try to get a hold of the echo and MRI, my shunt ration was 2:0.9 I think. I know it was just a tiny bit below. I have had a Holter monitor and done three months with an event recorder. They did the MRI because they had not been able to tell initially how many veins were involved from the echo, noticed the right side of my heart was noticeably bigger.

#

Hi,

Welcome back to icliniq.com.

Is your hemoglobin, chest X-ray normal, this is to make sure that your symptoms are related to heart condition only, an alternative reason for it. Now considering the shunt fraction of 2:0.9, which is equal to 2.2:1. Also, right-sided is enlarged as you mentioned, this becomes the indication for surgery. So I think surgery should be done in you.

For more information consult a cardiologist online --> https://www.icliniq.com/ask-a-doctor-online/cardiologist


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